September 26, 2009

Nicholas has been doing excellent for the past month. With only a few seizures last weekend. He had gone almost 4 weeks without a seizure prior. He is moving forward in physical therapy with great strengh and determination. He has started 1st grade at the Riddlebrook Elementry School. He loves his teacher and his two nurses. He gets off the bus each day with a big smile and “Mom I had a great day!” which is so peaceful to hear. And the reports from his nurses are that he is having a great time in class. He especially loves to be up front in his teachers hands involved in the days activities and assignments. He has been wearing his AFO’s more at school (for 2 1/2 hours a day ) to give him more support as well as using his walker which he calls a caboose.

first day of school

His eating continues to be stable, even better recently. He is slowly allowing his nurses to help him eat. He still does best eating when we are at a restaurant. Just this past weekend he was able to gobble up an adult size plate of 2 beef burritos smothered with a cup of sour cream. As a registered dietitian I feel that batten disease excellerates his caloric needs leaving him at higher nutritional risk. I have re-visited the choice to start the ketogenic diet and have continued to decline this route. My personal feeling is that he has a brain disease that is the cause of his epilepsy and a diet is not going to change his seizure severity or pattern. Nutrition is essential to maintaining brain function. His brain is under tremendous stress from this disease, therefore limiting his calorie intake in any way would not help his total body needs. I feel the best thing we do for Nicholas is push high calorie and high fat foods at every meal with high calorie snacks in between. I still feed him and encourage him to try to feed himself which he does with encouragement. It is a lot of work as he is not always interested in eating, easily distracted and sometimes eats while engaged in play. Sometimes he just feeds himself lots, when we show him where the food is (especialy steak!). I think that part of the issues is him not seeing where the food is. But what I do notice is that Nicholas does not clearly recognize when he is hungry or full. I have to really watch for cues of irritabilty (being hungry) and licking his lips often, turning his head away when he is full. I encourage him to use the “I am full” sign language to me when he is full by putting his hand up to his mouth to cover his mouth. Making sure he has consumed enough calories for the day is an important task and Nichoals depends on us.

Nicholas at PT

He is growing like a weed and has now has no front teeth!

Here is Nicholas on one of his first weeks of school. He especially loves being on the bus with other kids this year!

Nicholas at olde town day with one of his nurses Sarah

At his IEP meeting at his school with his team we have decided to do more with tactile, smells, and sound with Nicholas. This should make his experience at school more exciting, by helping Nicholas utilize and improve his strongest senses. Nicholas continues to be very social at school. Even with the children in the special needs room who have more difficulty communicating. Nicholas invites them to play with him… that is the Nicholas we know and love! I have to think that understanding what his needs are really help him be himself.

No new medicine changes yet this month but I do notice that in the evening Nicholas is slurring his words more than the morning time. Once he takes the Ativan his slurring is less and is more stable on his feet. I can see when the ativan is low in his body, he has more myoclonic jerks, will be hyperactive, has difficulty keeping with a task and is very irritable. Keeping his medication on a very strict schedule and closely monitoring symptoms we feel is key to keeping Nichoals feel like himself and happy. We are starting to wonder whether Ativan does something more than just prevent seizure activity.

Every fall the kids get excited for Halloween. Both Nicholas and William put on every costume we have… even if it is too small. Here is a great picture of the boys chasing their dad in the back yard as pirates. A great day for Nicholas.

Nicholas looking like a mean pirate!

Lastly we have found that the human gene therapy study has the possibility of being funded in the future. We are hopeful that this research will continue, but are very concerned that it will come too late for Nicholas and other children like him. We still need to do everything in our power to move forward and raise 2.5 million quickly. These kids with Batten disease decline at a rapid rate. So one child that is eligable now may decline too much over a period of just months to meet the study criteria. This study needs to be funded now, not at some distant date.

We just had a great fundraiser by the CKC Dance Acadamy in Hooksett NH. They had a CKC dance and zumba fitness “Totally 80’s night” fundraiser for us and raised the total of $660.00. The owners son Sam was in school with Nicholas when he was 4 years old and they were old buddies. Thank you Crystal Dionne for holding this fundraiser for us.!

Not So Plain Jane’s Salon in Manchester NH held another gene day for our promise to nicholas foundation. They raised $400.00 for our foundation. Thank you for you generous donation!

This last weekend we had Olde Town Day in Bedford NH. 1/3 of the proceeds from this event were raised for our foundation. We also received another $500 in direct donations at this event. Thank you to Betty, family and friends for helping us in this event to raise awarness for our foundation and our upcoming fundraiser events. It seems we are really getting the word out as most of the people I approached were well aware of our foundation.

Nicholas at olde town day.

UPCOMING FUNDRAISERS

Carrabbaas Italian Grill is currently selling “dinner on us” raffle tickets for two at their restaurants. The raffle tickets are $2 each. This will give one dinner per month for an entire year to the winner! Here is the contact information for the restaurant:

2 Upjohn St
Bedford, NH 03110-6705
(603) 641-0004

Thanks Skip!

Friday October 2, 2009 Our Promise To Nicholas Gala Manchester Country Club, Bedford NH. Tickets are on sale now. Please join us for an incredible event to benefit this foundation. A performance by The Boston Symphony Orchestra cellist, Luis Leguia. A rocking performance by The Jimmy Lehoux Band. Gourmet meal by Manchester Country Club, hundrends of scrumptious desserts donated by local bakerys. Silent Auction and raffle prizes to be won! http://promisetonicholasgala.weebly.com/ for more information. Contact: Heather @ heatherdainiak@hotmail.com or 603-617-2023 or Kim at knaioti@comcast.net

October Not so Scary Halloween Party and Movie Night in Bedford Saturday Oct 17, 2009– Location is on Rigley Field on the corner of Nashua and country Road in Bedford. Time of the event is 5PM-11PM. First movie starting at 6:30-7PM It’s the great Pumpkin Charlie Brown. Second Movie starts at 8:30PM is Beetle Juice. This event will be a halloween party. Kids wear costumes. Fall soups, drinks, popcorn, candy, donuts, hot apple cidar. Face painting, clowns and more! $5 at the door but donations are encouraged. If rains: Event will take place at Bedford High school. 100% of proceeds from this fundraiser go to Our Promise To Nicholas Foundation.

St. Anselm College Decathon for Our Promise To Nichoals Foundation– November 8, 2009.

Facebook Cause -Cure Genetic Brain Disease-Our Promise to Nicholas foundation. This cause is up and running thanks to my brother and sister in law Dan and Amy. So far it has 1,985 members and has raised $1,925.00. Please continue to send our cause to your friends and family to raise awareness to our foundation and batten disease. Together we can get closer to reaching our goal to start the gene therapy trial in NYC to save 5 children with Batten disease.